Background: To date, there are no studies associating the dried blood spot (DBS) levels of globotrioasylsphingosine (lysoGb3) with quantitative optical coherence tomography angiography (OCTA) parameters in Fabry disease (FD) patients. Here, we aimed to investigate the association between OCTA vessel density (VD), vessel length density (VLD) with DBS lysoGb3. Methods: A retrospective, single center analysis of all consecutive FD patients enrolled at the Department of Ophthalmology of the University Hospital of Zurich from December 1st, 2017 to September 9th, 2020. An association between VD and VLD detected by OCTA and lysoGb3 was investigated using a linear mixed model. Results: A total of 57 FD patients (23 male, 34 female; 109 eyes) were included. Forty-one patients suffered from the classic phenotype and 16 from the later-onset phenotype. LysoGb3 inversely correlated with VD and VLD in both the superficial (VD: p = 0.034; VLD: p = 0.02) and deep capillary plexus (VD: p = 0.017; VLD: p = 0.018) in the overall FD cohort. Conclusions: Our study shows an association between lysoGb3 and OCTA VD and VLD. This supports the hypothesis that quantitative OCTA parameters might be useful as diagnostic biomarkers for evaluating systemic involvement in FD, and possibly other diseases.

Whether post injectional acute intraocular pressure (IOP) increase is associated with decreased peripapillary and macular perfusion is still under debate. Here, we investigated early changes in the choroidal and retinal blood flow using OCTA imaging in a cohort of patients undergoing anti-VEGF intravitreal injections (IVI) for macular edema following retinal vein occlusion and diabetic retinopathy. In this prospective single-center, observational study, the pre- and post-IVI changes in retinal perfusion were examined via assessment of vessel length density (VLD) and vessel density (VD) in deep and superficial capillary segmentations (DCP and SCP), foveal avascular zone (FAZ) in SCP, as well as flow signal deficits in the choriocapillaris segmentation. Mean IOP significantly changed over the study course (p = 0.000; ANOVA). Measurements at 5 min post-IVI (33.48 ± 10.84 mmHg) differed significantly from baseline (17.26 ± 2.41 mmHg, p = 0.000), while measurements from one day, one week, and one-month post-IVI did not (p = 0.907, p = 1.000 and p = 1.000 respectively). In comparison to baseline, no changes in OCTA parameters, including FAZ, VD, VLD, and FV, were detected 5 min post-IVI. No significant alterations in OCTA parameters were observed during study course. Increased IOP spikes were detected post-IVI; however, no potential permanent ischemic retinal damage was suspected.

Background: During the first COVID-19 pandemic outbreak, a new triage concept had to be implemented for patients with retinal diseases having a scheduled appointment at the medical retina clinic. In this study, we aimed to assess patients’ confidence in this triage concept and patients’ satisfaction regarding the received treatment during the outbreak. Methods: This retrospective study included all patients with a diagnosed retinal disease, triaged into three priority groups based on their condition’s urgency during lockdown. After restrictions were eased, a subset of previously triaged patients was interviewed to assess their confidence in the triage and their satisfaction regarding the received treatment during the pandemic. Results: In total, 743 patients were triaged during the lockdown. Over 80% received an urgent appointment (priority 1). Among all priority 1 patients, over 84% attended their appointment and 77% received an intravitreal injection (IVI), while 7% cancelled their appointment due to COVID-19. In post-lockdown interviews of 254 patients, 90% trusted the emergency regimen and received treatment. Conclusions: Our triage seemed to be useful in optimizing access to treatment for patients with retinal diseases. An excellent rating of patients’ confidence in the triage and satisfaction regarding the received treatment during the first COVID-19 outbreak could be achieved.